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JACC Vol. 8, No.3
September 1986:621-6
621
Persistent Left Superior Vena Cava Communicating With the Left
Atrium Through a Systemic-Pulmonary Venous Malformation
DAVID S. LOOYENGA, MD, SAMUEL J. LACINA, MD, FACC, CARL J. GEBUHR, MD, FACC,
FRED S. STOCKINGER, MD
Grand Rapids, Michigan
A 14 year old white girl who presented with a brain
abscess was discovered to have a left pulmonary vascular
malformation on a chest roentgenogram. Angiograms
revealed a left superior vena cava that drained into a
venous malformation within the left lung, then communicated with the left atrium by way of the left superior
pulmonary vein. The right superior vena cava was func-
A persistent left superior vena cava is a rather common
anomaly occurring in approximately 0.3% (1,2) of the general population and in 4% (3-6) of patients with congenital
heart disease. This anomaly has frequently been reported
and is remarkable for its variation in anatomic presentation.
In the usual case (1,3-5) the superior venae cavae are bilateral and the left superior vena cava drains into the right
atrium through the coronary sinus (Fig. 1), thereby resulting
in normal hemodynamics. Approximately 17% (1,3) of patients with a persistent left superior vena cava have total
absence of the right superior vena cava. In approximately
10% of cases (3,4,7) the left superior vena cava terminates
in the left atrium. The anatomy of a left superior vena cavaleft atrial communication is either direct or through an initial
communication to a pulmonary vein.
In the case we report, the left superior vena cava drained
into a systemic-pulmonary venous malformation within the
left lung, and then communicated with the left atrium through
the left superior pulmonary vein. This case is presented
because we are unaware of a previously reported similar
case. The surgical correction and the resultant superior vena
cava syndrome are also unique to this case.
Case History
Clinical presentation. A 14 year old previously healthy
white girl presented with a 10 day history of progressive
From the Butterworth Hospital, Grand Rapids, Michigan.
Manuscript received December 13, 1985; revised manuscript received
March 24, 1986, accepted April 2, 1986.
Address for reprints: David S. Looyenga, MD, Medical Education
Department, Butterworth Hospital, 100 Michigan, Grand Rapids, Michigan 49503.
© 1986 by the American College of Cardiology
tionally absent and was anatomically an atretic cord.
There was mild systemic arterial hemoglobin desaturation, but no evidence of cyanosis. The embryology,
physiology and surgical repair of this rare lesion and the
complication of a postoperative superior vena cava syndrome are discussed.
(J Am Coil CardioI1986;8:621-6)
left-sided headache, blurry right-sided vision, low grade
fever, drowsiness and a stiff neck. The admission white
blood cell count was 15.5 x 103/mm3 but without a significant "left shift." The cerebral spinal fluid was slightly
turbid with 1,750 white blood cells, made up of 60% neutrophils and 40% lymphocytes. The patient was admitted
with a diagnosis of meningitis, but because of the focal
symptoms, a computed tomographic scan of the head was
performed which showed a left occipital brain abscess. The
admission chest roentgenogram (Fig. 2) revealed an abnormal vascular structure in the left lung field thought to be
either a pulmonary arterial-venous malformation or a vein
of anomalous pulmonary venous return. The cardiothoracic
ratio and heart configuration appeared norma!. The electrocardiogram revealed sinus bradycardia with a normal axis
and no evidence of chamber hypertrophy or strain. The
patient denied any history of cyanosis, fatigue, shortness of
breath, dyspnea on exertion or childhood pulmonary infection or growth problems. The physical examination of the
cardiovascular system was unremarkable.
The patient underwent a craniotomy with removal of the
abscess and its capsule. She was given antibiotic therapy
and recovered uneventfully. Cultures of the abscess grew
anaerobic Streptococcus intermedius. a bacterium that is
indigenous to the oral cavity and the gastrointestinal tract.
Diagnostic evaluation. One month after the craniotomy
the patient underwent further evaluation of the vascular
anomaly seen on the chest roentgenogram. Radial artery
blood gas determination at rest revealed a partial pressure
of oxygen of 64 mm Hg and an oxygen saturation of 92%.
The hemoglobin was 15.1 g/d!. Right brachial vein catheterization with dye injection into the right subclavian vein
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LOOYENGA ET AL.
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Figure 3. Angiogram of the left subclavian vein with opacification
of the distal left subclavian vein, the distal left jugular vein and
the anomalous vascular structure in the left lung seen on the chest
X-ray film (see Fig. 2).
Figure 1. Usual presentation of a persistent superior vena cava.
A. = aorta; c.s. = coronary sinus; I. V.c. = inferior vena cava;
L.A. = left atrium; L.S.V.c. = left superior vena cava; L.V. =
left ventricle; P.A. = pulmonary artery; R.A. = right atrium;
R.S.V.C. = right superior vena cava; R.V. = right ventricle.
opacified the distal right subclavian vein with retrograde
flow into the right jugular vein. The right superior vena cava
could not be opacified or catheterized. Dye in the right
jugular vein flowed to the left jugular vein through multiple
small anastomotic veins in the neck. Left brachial vein catheterization (Fig. 3) with dye injection into the left subclavian
vein opacified the distal left subclavian vein, the left jugular
vein and the vascular structure previously seen on the chest
roentgenogram. The patient's entire venous drainage of the
upper limbs and head was through a persistent left superior
vena cava into a systemic-pulmonary venous malformation
within the left lung. The venous malformation then drained
to the left atrium through the left superior pulmonary vein
Figure 4. The great vein anatomy of this patient. Innom. =
innominate vein; Lt. Jug. = left jugular vein; Lt. Sc. = left
subclavian vein; Lt. SVC = left superior vena cava; Pulm, =
pulmonary; Rt. Jug. = right jugular vein; Rt. sc. = right subclavian vein; other abbreviations as in Figure I.
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Figure 2. Admission chest X-ray film revealing the anomalous
vascular structure in the left lung field.
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lACC Vol. 8. No.3
September 1986:621-6
LOOYENGA ET AL.
PERSISTENT LEFf SUPERIOR VENA CAVA
(Fig. 4). The remnant of the embryonic right superior vena
cava was an atretic cord seen later at thoracotomy.
Surgical correction. Because the patient was a candidate for a recurrent embolic event, surgical amelioration of
the right to left shunt was attempted. The anomalous vessel
was dissected from the venous malformation and was connected to the left atrial appendage. This in tum was diverted
under a Gortex baffle, across the left atrium, through the
intraatrial septum and into the right atrium.
One day after the surgical correction. the patient had
the onset of progressive edema of the head and upper limbs.
The diagnosis of a superior vena cava syndrome was entertained and confirmed by angiography (Fig. 5A). The pressure was 22 mm Hg in the left superior vena cava and 6
mm Hg in the right atrium, resulting in a mean pressure
gradient across the stenotic site of 16 mm Hg. The stenosis
resulted from narrowing of the left atrial appendage tunnel.
This stenosis was specifically exacerbated by atrial systole.
Bilateral pulmonary artery angiography was also per-
Figure 5. Left superior vena cava angiograms. A, Performed after
the first median sternotomy showing the stenosis at the site of the
left atrial appendagetunnel. B, Performedafter the second median
sternotomy revealing full patency of the left superior vena cava,
left atrial appendage tunnel and the Gortex baffle to the right
atrium.
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623
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Figure 6. Great vein anatomy after surgical correction. Note the
Gortex baffle which extends through the left atrium and the intraatrial septum. The Gortex patch was needed to relieve stenosis in
the left atrial appendagetunnel(baffle). Abbreviations as in Figures
I and 4.
formed at this time. Injection into the left pulmonary artery
revealed a mildly congested arterial phase; however, the
venous phase was significantly delayed with slow progressive opacification of the venous malformation, which was
emptied by the left superior pulmonary vein into the left
atrium. The left inferior pulmonary vein emptied into the
malformation as well. Right pulmonary artery angiography
was normal. The mean pulmonary artery pressure measured
before angiography was 46125 mm Hg. Angiography of the
aorta revealed entirely normal arterial anatomy.
The patient underwent a second median sternotomy. The
left atrial appendage was opened lengthwise and a Gortex
patch was placed in the incision, thus ensuring patency of
the tunnel. A postoperative angiogram (Fig. 5B) showed a
widely patent vessel from the left superior vena cava to the
right atrium. A schematic representation of the postsurgical
anatomy is seen in Figure 6. The patient recovered uneventfully and continues to be asymptomatic.
Discussion
Reported cases. The anomaly of persistent left superior
vena cava is remarkable for its number of anatomic varia-
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LOOYENGA ET AL.
PERSISTENT LEFT SUPERIOR VENA CAVA
tions. In general, only cases with abnormal drainage and
resultant right to left shunting are of clinical significance.
The reported incidence of left superior vena cava terminating
in the left atrium is approximately 10% (3,4,7). In the majority of these cases, the right superior vena cava is also
present and drains normally. Nearly all cases of left atrialleft superior vena cava communication have been associated
with significant intracardiac anomalies (1,6,8,9); in more
than 95% (1, 10,11) there is an anomaly of the atrial septum
(atrial septal defect, common atrium or common atrioventricular canal). Other associated anomalies (8,10,11) include
anomalous pulmonary venous retum, coarctation of the aorta,
dextrocardia, inferior vena cava abnormalities, patent ductus
arteriosus, pulmonary stenosis, tetralogy of Fallot, transposition of the great arteries and tricuspid atresia. A few
cases of isolated left atrial-left superior vena cava communication have been reported (12); however, most of these
cases have a normally draining right superior vena cava as
well. Only two cases of isolated left atrial-left superior vena
cava communication with absent right superior vena cava
have been reported (13,14). Tuckman et al. (13) reported
the case of a 15 year old boy who presented with fatigue,
fingertip cyanosis, polycythemia and left ventricular hypertrophy. Sherafat et al. (14) described a 5 year old boy
who presented with a cerebral abscess who also had mild
cyanosis and clubbing of the fingers and toes.
Types of left atrial-left superior vena cava connections. Three types of these connections have been described
(6,10). Most frequently, the connection is direct, with the
site of entrance near that of the pulmonary vein. This type
is usually associated with a normal coronary sinus. The
second type of connection occurs when the ostium of the
coronary sinus fails to develop, resulting in blood from both
the left superior vena cava and the coronary sinus entering
into the left atrium. The third type of connection is through
an anastomosis with a pulmonary vein which then drains
into the left atrium. This is usually associated with a normal
coronary sinus.
Figure 7. Normal embryology of the superior vena cava system.
A, The primitive heart with paired subclavian veins and paired
veins draining the primitive lung buds into the bilateral anterior
cardinal veins. B, Development of the innominate vein between
the bilateral anterior cardinal veins and the bilateral pulmonary
veins draining the enlarging pulmonary vasculature. The shaded
vessel is the left superior vena cava. C, Obliteration of the left
superior vena cava and the veins that initially drained the lung
buds (dashed lines). D, Normal great vein anatomy.
lACC Vol. 8, No.3
September 1986:621-6
An important differentiation must be made between a left
atrial-left superior vena cava communication and a levoatriocardinal vein as it was initially described by Edwards
and DuShane (15). The latter vessel is also due to the persistence of the primordial lung bud splanchnic plexus and
the left cardinal vein. However, the directional flow of blood
in the levoatriocardinal vein is opposite to that of a left
atrial-left superior vena cava communication. In a levoatriocardinal vein the blood flows from the pulmonary vein
or left atrium to the persistent left superior vena cava and
then to the innominate vein, resulting in a left to right shunt
rather than a right to left shunt.
Embryologic considerations. The embryologic development of the systemic and pulmonary circulations occurs
concurrently and very early (4,5,7,8,16,17). Blood within
the embryo is returned to the primitive heart through the
paired anterior and posterior cardinal veins (Fig. 7A). The
anterior cardinal veins drain the cephalad portions of the
embryo and the upper limbs through the bilateral subclavian
veins. Also draining into the cardinal veins is a portion of
the splanchnic plexus of the developing foregut and the
associated primitive lung buds (Fig. 7A).
With subsequent development in the second embryonic
month, the major changes are: 1) the development of a
channel (left innominate vein) between the paired anterior
cardinal veins, which shunts the blood primarily to the right
side (Fig. 7B); 2) the development of a direct communication (pulmonary veins) between the left atrium and the
rapidly enlarging pulmonary vasculature (Fig. 7B); and 3)
with preferential flow to the right anterior cardinal vein and
preferential drainage of the lung through the bilateral pulmonary veins, the left cardinal vein and the veins that initially drained the lung buds become obliterated (Fig. 7C).
Further development results in normal anatomy with a right
superior vena cava and bilateral pulmonary veins which later
become four in number as the proximal aspect of these veins
becomes incorporated into the posterior wall of the left
Figure 8. Presumed embryology of this patient's superior vena
cava system. A, Normal early development. B, Development of
the innominate vein and the pulmonary veins. C, Deviation from
normal with obliterationof the right anterior cardinal vein and the
middle portion of the left anterior cardinal vein (dashed lines).
Blood from the cephalad portion of the embryo returns to the heart
through reverse flow into the splanchnic plexus, becoming congested and forming an intrapulmonary venous malformation, D,
This patient's superior vena cava anatomy with drainage of the
malformation into the left atrium through the superior pulmonary
vein,
A
B
o
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September 1986:621-6
atrium. The very proximal portion of the left anterior cardinal vein becomes the coronary sinus and the oblique vein
of the left atrium.
The embryologic development of this patient is presumed
to have occurred as follows (Fig . 8). Early embryologic
development, including the development of the left innominate vein and the pulmonary veins , was normal (Fig. 8A
and B). Deviation from normal occurred when both the right
anterior cardinal vein and the middle portion of the left
anterior cardinal vein became slowly obliterated (Fig. 8C).
Blood from the cephalad portion of the embryo returned to
the heart through the splanchnic plexus of the left lung (Fig.
8C). This venous plexus became congested and enlarged,
forming a venous malformation that ultimately drained through
the left superior pulmonary vein into the left atrium. A
diagram of this patient's superior great vein anatomy is seen
in Figure 80. It is of interest that the coronary sinus in this
patient is normal.
Hemodynamic considerations. Because the intracardiac anatomy was normal, the only hemodynamic abnormality was that of the left atrial-left superior vena cava
communication and the resultant right to left shunt. The
patient's initial presentation with a cerebral abscess was
secondary to a paradoxical septic embolus, presumably from
an asymptomatic oral infection. A cerebral abscess is a well
documented (3,14) complication of this type of right to left
shunt.
Cyanosis. Although this patient had mild hemoglobin
desaturation, the lack of cyanosis is somewhat surprising ,
considering the calculated pulmonary blood flow to systemic
blood flow ratio of approximately 0.66 . This calculation is
dependent on the assumption that the superior vena cava
normally returns approximately 33% of the venous return
(I8) , Many of the previous cases of left atrial-left superior
vena cava communication (6,12,18,19) have been associated with cyanosis; however, the origin of the cyanosis is
often nebulous in light of frequent severe intracardiac anomalies, Meadows and Sharp (9) proposed five mechanisms
that determine the degree of cyanosis in left atrial-left superior vena cava communication . Four of these mechanisms
involve the relative flow dynamics of the right and left
superior venae cavae. However, because this patient did not
have a right superior vena cava, these mechanisms do not
apply. The fifth mechanism involves the presence of anastomotic vessels that ultimately drain to the right atrium,
therefore minimizing the right to left shunt. The angiograms
performed after the initial surgery revealed several small
vessels from the left superior vena cava that appeared to
communicate with the hemiazygos system and the paraspinal venous plexus. The extent to which this minimized the
shunt preoperatively is difficult to assess .
Surgical repair. Surgical repair of the right to left shunt
was necessary to avoid potential recurrence of embolic phenomena (1 ,7) and to avoid hemodynamic deterioration that
LOOYENGA ET AL.
PERSISTENT LEFf SUPERIOR VENA CAVA
625
could occur with time (II). The transfer of the anomalous
vessel from the left side (in this case from the left systemicpulmonary venous malformation) to the right atrium involves two considerations (8). First, adequate length of the
anomalous vessel must be attained to reach the right atrium ,
and second, tension on the anomalous vessel must be minimized to avoid stenosis. These two considerations were
met with the use of a Gortex baffle through the cavity of
the left atrium. The postoperative stenosis of the left atrial
appendage tunnel resulting in superior vena cava obstruction
was easily ameliorated with the use of a second Gortex patch
at the stenotic site.
We gratefully acknowledge the secretarial assistance of Celia M. Steele.
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